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  What is Congenital Diaphragmatic Hernia (CDH)?

CDH is a developmental defect in the diaphragm (commonly on the left side), which occurs in 1 per 2200 births. CDH occurs when a fetus’ intestines and other abdominal organs develop within the chest cavity during pregnancy. This restricts the ability of the fetus’ heart and lungs to grow. Infants born with CDH suffer from underdeveloped lungs (pulmonary hypoplasia) and high blood pressure in their lungs (pulmonary hypertension). They may need high levels of heart and lung support, which includes extra­corporeal membrane oxygenation (ECMO), a device that circulates blood through a machine so that it can be oxygenated. Surgical treatment involves moving the abdominal organs back into the abdomen. This is followed by closing the hole in the diaphragm through which the abdominal organs passed. The outcome depends mostly on the severity of the infant’s pulmonary hypoplasia and pulmonary hypertension. The best current estimate of after-birth survival rate is 63%. However, infants who survive after birth without any major complications should have a good long-term outcome because of the lungs’ growth potential.

CDH was once thought to need immediate surgical treatment. However, over the past two decades, physicians have favored treatment plans that involve initial respiratory "stabilization" and delayed surgery. The change in practice followed the release of a Canadian report, which showed that delayed surgery and immediate surgery result in comparable outcomes for infants with CDH. Many hospitals that use delayed surgical have reported improved survival rates in their patients. However, some studies do not show that there is a higher chance of survival with delayed surgery. Another recent form of treatment has been the use of less aggressive ventilator (breathing) strategies, so that lung injuries decrease in patients with CDH. Some studies have reported that using less aggressive treatment results in improved outcomes, while other studies have not. Other controversial therapies involve using inhaled nitric oxide and other medications to decrease high blood pressure in an infant’s lungs, using steroids and surfactant, using ECMO and the timing of surgical repair in relation to ECMO, and the use of new ventilation methods. For all of these treatments, clinical practices vary greatly from one hospital to another.

 

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